目的研究Ｗｉｌｓｏｎ病（ＷＤ）患者和非ＷＤ对照者胆汁铜、锌含量的变化，结合临床表型进一步探讨ＷＤ铜滞留的病因机制．方法不同分型、病情各异的ＷＤ患者２０例，有慢性肝损害的非ＷＤ患者２２例和健康自愿者１０例，均实施十二指肠引流术留取胆汁样本，采用原子吸收分光光度计检测各样本的铜、锌含量．结果ＷＤ患者的胆汁铜含量（μｍｏｌ／Ｌ，４４±０４ｖｓ４１７±２０，４２０±２６）和铜／锌比值（０１３±００２ｖｓ１５４±０２７，１５６±０２４）显著低于有慢性肝损害的非ＷＤ患者及健康自愿者（Ｐ＜００１），而胆汁锌含量无明显差异（Ｐ＞００５）．不同分型和病情状况的ＷＤ患者胆汁铜含量存有显著性差异（Ｐ＜００５；Ｐ＜００１）．结论肝胆系统铜排泄显著减少是ＷＤ患者铜滞留的关键，胆汁铜滞留与ＷＤ患者肝损害的程度和病情轻重有密切关系． Objective To study the changes of bile copper and zinc contents in patients with Wilson’s disease (WD) and non-WD controls and to explore the etiological mechanism of WD copper retention in combination with clinical phenotypes. Methods 20 cases of WD patients with different types and different conditions, 22 cases of non-WD patients with chronic liver damage and 10 healthy volunteers were enrolled in this study. All patients underwent biliary drainage with duodenal drainage and were analyzed by atomic absorption spectrophotometer Detection of the samples of copper, zinc content. Results In patients with WD, the contents of bile copper (μmol / L, 44 ± 04 vs 417 ± 20, 420 ± 26) and copper / zinc ratio (013 ± 002 vs154 ± 027,156 ± 024) were significantly lower than those of non-WD patients with chronic liver damage and healthy volunteers (P <001), while there was no significant difference in bile zinc content (P> 005). There was a significant difference in the contents of bile copper in WD patients with different types and conditions (P <005; P <001). Conclusion The significant reduction of copper excretion in hepatobiliary system is the key to the copper retention in WD patients. The retention of bile copper is closely related to the degree of liver damage and severity of disease in patients with WD.